Abstract:
:The ability to deliver enzymatically active human hexosaminidase A across the blood-brain barrier and into brain cells of the normal rat was examined. Following osmotic blood-brain barrier modification in the rat, intraarterially administered human hexosaminidase A and B were shown to cross the barrier and enter brain cells. Subcellular fractionation studies demonstrated that most of the human enzyme delivered across the barrier was functionally active and appeared to be inside a subcellular organelle. These studies provide evidence that blood-brain barrier modification permits delivery of functionally active hexosaminidase A into subcellular organelles consistent with that known to be the appropriate site of physiologic activity.
journal_name
Neurologyjournal_title
Neurologyauthors
Neuwelt EA,Barranger JA,Pagel MA,Quirk JM,Brady RO,Frenkel EPdoi
10.1212/wnl.34.8.1012subject
Has Abstractpub_date
1984-08-01 00:00:00pages
1012-9issue
8eissn
0028-3878issn
1526-632Xjournal_volume
34pub_type
杂志文章相关文献
NEUROLOGY文献大全abstract:OBJECTIVE:To examine the association between socioeconomic status (SES) and disability outcomes and progression in multiple sclerosis (MS). METHODS:Health administrative and MS clinical data were linked for 2 cohorts of patients with MS in British Columbia (Canada) and South East Wales (UK). SES was measured at MS sym...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/WNL.0000000000007190
更新日期:2019-03-26 00:00:00
abstract:OBJECTIVE:To explore the inflammatory processes in the pathogenesis of psychiatric symptoms and the prognostic value of psychiatric comorbidities in multiple sclerosis (MS). METHODS:Four hundred five patients with relapsing-remitting (RR) MS underwent psychiatric evaluation by means of Beck Depression Inventory II (BD...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/WNL.0000000000004411
更新日期:2017-09-26 00:00:00
abstract:OBJECTIVE:To compare characteristics between Black and White patients with amyotrophic lateral sclerosis (ALS) in order to identify disparities and phenotypic variability. METHODS:We performed database review for patients seen between 1997 and 2020 at the Emory ALS Center in Atlanta, Georgia. Patients with ALS were in...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/WNL.0000000000011396
更新日期:2020-12-28 00:00:00
abstract:OBJECTIVES:SCN1A mutations were recently reported in several patients with severe myoclonic epilepsy in infancy (SMEI). The authors analyzed SCN1A mutations in 93 patients with SMEI and made genotype-phenotype correlation to clarify the role of this gene in the etiology of SMEI. METHODS:All patients fulfilled the crit...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/01.wnl.0000069463.41870.2f
更新日期:2003-06-24 00:00:00
abstract:OBJECTIVES:More than 30 different rare mutations, including copy number variants (CNVs), in the amyloid precursor protein gene (APP) cause early-onset familial Alzheimer disease (EOFAD), whereas the contribution of common APP variants to disease risk remains controversial. In this study we systematically assessed the r...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/WNL.0b013e3182515972
更新日期:2012-04-17 00:00:00
abstract:OBJECTIVE:We sought to examine brain white matter abnormalities based on MRI in adults with Lesch-Nyhan disease (LND) or an attenuated variant (LNV) of this rare, X-linked neurodevelopmental disorder of purine metabolism. METHODS:In this observational study, we compared 21 adults with LND, 17 with LNV, and 33 age-, se...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/WNL.0000000000001128
更新日期:2015-01-13 00:00:00
abstract:OBJECTIVES:We used functional MRI (fMRI), transcranial Doppler ultrasound, and visual evoked potentials (VEPs) to determine the nature of blood flow responses to functional brain activity and carbon dioxide (CO2) inhalation in patients with cerebral amyloid angiopathy (CAA), and their association with markers of CAA se...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/01.wnl.0000435291.49598.54
更新日期:2013-11-05 00:00:00
abstract::We report imaging and clinical findings of leukoencephalopathy occurring after levamisole. The lesions were hypoattenuating on CT and appeared as multifocal oval or elliptical foci in the white matter or along the subependymal veins on MRI. Most lesions resolve after prompt withdrawal of levamisole. Detailed history t...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/01.wnl.0000237344.06122.79
更新日期:2006-09-26 00:00:00
abstract::Endovascular therapy for acute ischemic stroke continues to evolve to improve both efficacy and safety. In the late 1990 s, intra-arterial chemical thrombolysis with prourokinase was shown to be effective in achieving partial recanalization and improving clinical outcome, in comparison with intra-arterial heparin admi...
journal_title:Neurology
pub_type: 杂志文章,评审
doi:10.1212/WNL.0b013e3182697e9e
更新日期:2012-09-25 00:00:00
abstract::All Olmsted County, Minnesota, residents who experienced brain injury from 1935 through 1979 were identified and their medical records reviewed for survival and neurologic outcome. Minimum inclusion criteria included loss of consciousness or post-traumatic amnesia or neurologic evidence of brain injury or skull fractu...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.39.1.96
更新日期:1989-01-01 00:00:00
abstract:OBJECTIVE:To determine whether comorbidity is associated with clinical (relapses, disability worsening) and MRI outcomes in multiple sclerosis (MS) by conducting a secondary analysis of the CombiRx clinical trial. METHODS:CombiRx compared interferon beta-1a, glatiramer acetate, and the combination of these agents. For...
journal_title:Neurology
pub_type: 杂志文章,随机对照试验
doi:10.1212/WNL.0000000000010024
更新日期:2020-08-04 00:00:00
abstract:BACKGROUND:Basic issues regarding factors influencing progressive supranuclear palsy (PSP) patient caregiver burden remain unresolved, including whether and how disease severity and duration influence caregiver burden. OBJECTIVE:To examine the relation between PSP patient caregiver burden and disease severity, describ...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.51.5.1303
更新日期:1998-11-01 00:00:00
abstract:BACKGROUND:Although hospital-outcome relationships have been explored for a variety of procedures and interventions, little is known about the association between annual stroke admission volumes and stroke mortality. Our aim was to determine whether facility type and hospital volume was associated with stroke mortality...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/01.wnl.0000268485.93349.58
更新日期:2007-09-11 00:00:00
abstract::A 61-year-old woman presented with two paraneoplastic neurologic disorders--Lambert-Eaton myasthenic syndrome (LEMS) and subacute cerebellar degeneration (SCD)--that antedated the diagnosis of small-cell carcinoma of the lung by 15 months. Plasmapheresis initiated before the identification of the tumor had a beneficia...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.41.10.1682
更新日期:1991-10-01 00:00:00
abstract::About one-third of patients with acoustic tumor (AT) initially seek medical attention for nonaudiologic complaints. The nonspecific early symptoms of AT require the inclusion of AT in many neurologic differential diagnoses. Advances in electrophysiologic and radiographic tests have allowed earlier diagnosis of AT at a...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.33.2.211
更新日期:1983-02-01 00:00:00
abstract::A 52-year-old steelworker displayed a consistent defect in leftward voluntary gaze across the midline. Both fast (saccadic) and slow (pursuit) eye movements were affected. Neuro-ophthalmologic evaluation revealed no additional neurologic or ophthalmologic deficit. Pupilary miosis occurred during leftward gaze attempt,...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.29.1.82
更新日期:1979-01-01 00:00:00
abstract:BACKGROUND:Cerebral microbleeds are known to be indicative of bleeding-prone microangiopathy and may predict incident intracerebral hemorrhage (ICH). In this study, we investigated whether microbleeds are associated with the incidence of warfarin-related ICH. METHODS:Twenty-four patients with ICH while on outpatient t...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/01.wnl.0000339060.11702.dd
更新日期:2009-01-13 00:00:00
abstract::Clinicians have long suspected an association of classic amyotrophic lateral sclerosis (ALS) with Parkinson's disease (PD), dementia, or both. If proven, this would raise the possibility of a shared genetic susceptibility to the three disorders. To investigate this hypothesis, we compared 151 newly diagnosed ALS patie...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.44.10.1872
更新日期:1994-10-01 00:00:00
abstract::Single motor unit firing rates were measured from the first dorsal interosseous muscle (FDI) of normal subjects and patients with partial denervation of that muscle. Motor unit discharges were recorded at various levels of voluntary, stationary, isometric contraction of the FDI. The mean increase in firing rate associ...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.28.12.1241
更新日期:1978-12-01 00:00:00
abstract::We studied the effect of CSF drainage on cerebral blood flow (CBF) in normal pressure hydrocephalus (NPH) and non-NPH dementia using the 133Xenon inhalation technique. Dementia patients had lower CBF than matched elderly normals. Flow values for NPH and non-NPH patients did not differ before or after CSF drainage. CBF...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.34.1.96
更新日期:1984-01-01 00:00:00
abstract:OBJECTIVE:To compare the accuracy of Alberta Stroke Program Early Computed Tomography Score (ASPECTS) and CT perfusion to detect established infarction in acute anterior circulation stroke. METHODS:We performed an observational study in 59 acute anterior circulation ischemic stroke patients who underwent brain noncont...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/WNL.0000000000004028
更新日期:2017-06-13 00:00:00
abstract::The authors studied immunoglobulin (Ig) G subclasses in the CSF and sera of patients with relapsing neuromyelitis optica (RNMO) and typical multiple sclerosis (MS). Although the total IgG concentrations were elevated in the CSF of patients with RNMO and MS, IgG1% and IgG1 index were significantly elevated only in pati...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.62.1.144
更新日期:2004-01-13 00:00:00
abstract::Current concepts suggest that avoidance of pulsatile stimulation of dopamine receptors in Parkinson's disease (PD) can prevent the onset of dyskinesia. In MPTP-treated primates, repeated administration of levodopa or other short-acting dopamine agonist drugs leads to the onset of marked involuntary movements. In contr...
journal_title:Neurology
pub_type: 杂志文章,评审
doi:10.1212/wnl.62.1_suppl_1.s47
更新日期:2004-01-13 00:00:00
abstract:BACKGROUND:The APOE epsilon4 allele has emerged as a major genetic factor for Alzheimer disease (AD), and its presence has been associated with an increase in beta-amyloid senile plaques (SPs) and neuritic plaques (NPs). Whether it affects neurofibrillary tangle (NFT) accumulation or cholinergic losses in AD remains co...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/01.wnl.0000128091.92139.0f
更新日期:2004-06-08 00:00:00
abstract::The authors found serum immunoglobulin M (IgM) autoantibody in a patient with typical acute cerebellar ataxia (ACA) and identified the antigen molecule as triosephosphate isomerase (TPI). TPI antigenicity to the patient's antibody was the highest in the cerebellar tissue. Eight of 23 patients with ACA had increased Ig...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/01.wnl.0000178802.38268.1e
更新日期:2005-10-11 00:00:00
abstract::Erythrocyte calcium transport as a function of free [Ca++] was examined using inside-out vesicles from eight pairs of patients with Duchenne dystrophy and age- and sex-matched controls. The Km for Ca++ of the transport protein and the Vmax were higher in the Duchenne vesicles in seven of the eight pairs. The mean Km a...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.30.11.1236
更新日期:1980-11-01 00:00:00
abstract::We describe a 78-year-old man with right hemiballism as the presenting symptom of a metastatic occult adenocarcinoma of the lung. CT demonstrated two contrast-enhancing lesions: one in the left subthalamic region and the other in the left parietal region. Metastatic tumor was confirmed at autopsy. ...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.34.2.204
更新日期:1984-02-01 00:00:00
abstract::The authors describe a patient with bilateral papilledema, visual field abnormalities, poorly reactive pupils, meningeal enhancement on cranial MRI, and diffuse brain parenchymal hypervascularity. The opening pressure at the time of lumbar puncture was normal, and results of other CSF studies were normal. All abnormal...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.53.9.2204
更新日期:1999-12-10 00:00:00
abstract::The effect of imipramine on the absorption of a single dose of levodopa was studied in male volunteers. By delaying gastric emptying and retarding delivery to intestinal absorptive sites, imipramine interfered with the absorption of levodopa. This action caused by the anticholinergic effect of imipramine. The retardat...
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.25.11.1029
更新日期:1975-11-01 00:00:00
abstract:OBJECTIVES:To describe the clinical, CSF, and radiologic findings and long-term follow-up in a cohort of patients with acute disseminated encephalomyelitis (ADEM), and to determine possible prognostic factors for progression to MS. METHODS:Forty adults (28 women, mean age 33.5 years) diagnosed with ADEM were analyzed....
journal_title:Neurology
pub_type: 杂志文章
doi:10.1212/wnl.56.10.1313
更新日期:2001-05-22 00:00:00